Aggressive Digital Papillary Adenocarcinoma: A Meta-Analysis of the Presentation, Treatment, and Outcomes of a Rare Hand Tumor

Background: Aggressive digital papillary adenocarcinoma (ADPA) is a rare skin adnexal tumor with a predilection for the hand. The presentation, treatment, and outcomes of ADPA remain poorly defined due to the scarcity of reports and low-level evidence of published findings.

Methods: We performed a meta-analysis following Preferred Reporting Items for Systematic reviews and Meta-Analyses guidelines with the intent to provide hand surgeons a better understanding of the diagnosis and treatment of the disease. Three additional cases of ADPA from our institution were combined with the results from the systematic review.

Results: A total of 238 cases were included in the meta-analysis. Most ADPAs were painful, evolving, and less than 2 cm in size. Regional sentinel lymph node biopsies were performed in 55 patients, of which 25.5% were positive. Overall cohort recurrence and distant metastasis rates were 24.3% and 18.1%, respectively. Patients who obtained clear margins within 6 months of diagnosis had significantly lower rates of recurrence, metastasis, and mortality (P < .001, P = .04, P = .003, respectively). There were no differences in recurrence or metastasis rates in those who obtained clear margins by excision or amputation (P = .27 and P = .07, respectively). In patients who died from disease (6.0%), 5.0 years was the average time to death.

Conclusions: Aggressive digital papillary adenocarcinoma should be included in the differential diagnosis of hand lesions with unusual presentations or lack of response to other therapies. Clear margins should be obtained within 6 months of diagnosis, and routine surveillance should be provided by an oncologic physician familiar with ADPA as these tumors have high recurrence rates.